Webb Alastair John Stewart, Conlon Chris, Briley Dennis
Department of Clinical Neurosciences, University of Oxford, Oxford, UK.
Pract Neurol. 2012 Oct;12(5):324-7. doi: 10.1136/practneurol-2012-000269.
A 60-year-old man presented with an acute, pruritic, erythematous rash associated with marked hypereosinophilia (2.34×10(9)/l (0.04-0.40)). There was eosinophilic infiltration on hepatic, bone marrow and lymph node biopsies, with multiple lung nodules and mild splenomegaly. However, extensive investigation excluded parasitic or bacterial causes, specific allergens or the Fip1L1 mutation seen in myeloproliferative hypereosinophilia. Six months into the illness, he developed an acute, left, complete lower motor neurone facial palsy over hours, and an acute right lower motor neurone facial palsy 2 weeks later, without recovery. Over the subsequent 3 months, he developed complex partial seizures, a transient 72-h non-epileptic encephalopathy and episodic vertigo with ataxia. Further investigation showed bilateral enhancement of the VII nerves and labyrinthis on gadolinium-enhanced MR brain scan, cerebrospinal fluid lymphocytosis and neurophysiological evidence of polyradicolopathy. His eosinophil count fell with corticosteroids, hydroxycarbamide, imatinib and ultimately mepolezumab, but without symptomatic improvement. Repeat lymph node biopsy showed Kaposi's sarcoma, leading to a diagnosis of HIV-1 infection with a modestly reduced CD4 count of 413×10(6)/l (430-1690). Hypereosinophila and eosinophilic folliculitis are recognised features of advanced HIV infection, and transient bilateral facial palsy occasionally occurs at the time of seroconversion. This is the first report of a chronic bilateral facial palsy likely due to primary HIV infection, not occurring during seroconversion and in association with hypereosinophilia. This case emphasises the protean manifestations of HIV infection and the need for routine testing in atypical clinical presentations.
一名60岁男性出现急性、瘙痒性、红斑性皮疹，并伴有明显的嗜酸性粒细胞增多（2.34×10⁹/L（0.04 - 0.40））。肝、骨髓和淋巴结活检显示有嗜酸性粒细胞浸润，伴有多个肺结节和轻度脾肿大。然而，广泛检查排除了寄生虫或细菌病因、特定过敏原或在骨髓增殖性嗜酸性粒细胞增多症中所见的Fip1L1突变。患病6个月时，他在数小时内出现急性左侧完全性下运动神经元性面瘫，2周后又出现急性右侧下运动神经元性面瘫，且未恢复。在随后的3个月里，他出现了复杂部分性发作、持续72小时的短暂非癫痫性脑病以及伴有共济失调的发作性眩晕。进一步检查显示，钆增强磁共振脑扫描显示双侧面神经和内耳增强，脑脊液淋巴细胞增多以及多神经根病的神经生理学证据。他的嗜酸性粒细胞计数在使用皮质类固醇、羟基脲、伊马替尼以及最终使用美泊利单抗后下降，但症状无改善。重复淋巴结活检显示为卡波西肉瘤，从而诊断为HIV - 1感染，CD4计数略有下降，为413×10⁶/L（430 - 1690）。嗜酸性粒细胞增多和嗜酸性毛囊炎是晚期HIV感染的公认特征，血清转化时偶尔会出现短暂的双侧面瘫。这是首例可能由原发性HIV感染导致的慢性双侧面瘫的报告，并非发生在血清转化期间，且与嗜酸性粒细胞增多有关。该病例强调了HIV感染的多种表现形式以及在非典型临床表现中进行常规检测的必要性。
